TAILIEUCHUNG - Review article Anaesthetic management of the child with sickle cell disease

Parents with Parkinson’s agree that you shouldn’t try to hide what you’re going through from your family. Doing so can backfire on you. “It used to be that when I’d have my ‘off’ periods, I would go into my room rather than subject my family to it,” says Joan S. “That was a big mistake, because they needed to see what I was going through in order to understand.” Greg H. has had two successful rounds of DBS surgery since his diagnosis in 1990. * He says his kids have become very sensitive to his needs when he goes in for what he calls his annual “stimulator appreciation day” where the. | Paediatric Anaesthesia 2003 13 473-489 Review article Anaesthetic management of the child with sickle cell disease WARWICK A. MARCHANT BSc MBBS FRCA AND ISABEAU WALKER BSc MBBChir FRCA Specialist Registrar and Consultant in Anaesthesia Great Ormond Street Hospital for Children London UK Summary Sickle cell disease SCD is a relatively common inherited disorder of haemoglobin with significant morbidity and mortality. This review describes the epidemiology and pathophysiology of the disease and discusses the clinical manifestations found in children with SCD. A discussion of the evidence concerning the perioperative management of such children is presented. Keywords Haemoglobin Sickle Anaemia Sickle cell Perioperative care Paediatrics Introduction Sickle cell disease SCD is part of a heterogeneous group of inherited disorders of the p-haemoglobin chain characterized by chronic haemolytic anaemia intermittent vaso-occlusive crises and marked variability in the severity of disease between individuals. It is a multisystem disorder the acute and chronic manifestations of which are of relevance to the anaesthetist. Normal adult red blood cells contain three different types of haemoglobin. Haemoglobin A HbA is formed by two a and two p globin chains a2p2 and comprises 96-98 of total haemoglobin. Haemoglobin A2 HbA2 made up from two a and two s globin chains a2s2 accounts for and fetal haemoglobin HbF made up from two a and two c globin chains a2y2 for 1 . Until the first 10 weeks of life HbF comprises up to 90 of total haemoglobin. Correspondence to Dr Warwick A Marchant Specialist Registrar in Anaesthesia Great Ormond Street Hospital for Children Great Ormond Street London WC1N 3JH email . Haemoglobin S HbS contains an abnormal p globin chain because of the inheritance of the sickle p globin gene on chromosome 11. Valine is substituted for glutamic acid in the sixth position of the p globin chain. The mutation appears to be .

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